Eshita Khan

and 1 more

Abstract: Objective: Pediatric acute promyelocytic leukemia (APL) is one of the most curable subtypes of acute myeloid leukemia of childhood. But it may have many early complications, especially in developing countries. This study aims to describe the early course of disease and adverse events in the management of pediatric APL cases in Bangladesh. Method: This prospective observational study was conducted in the Department of Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University, Dhaka from September 2017 to March 2019. The study included twenty PML:RAR-α (ProMyelocytic Leukemia-Retinoic Acid Receptor-α) positive APL cases. After taking informed written consent from the parents, patients were treated with risk directed ATRA (All-trans-retinoic acid) based chemotherapy. Results: The mean age was 6.8 years with male-female ratio of 1:1.2. Hemorrhagic manifestations were observed in 95% of patients, with mucosal bleeding in 55% and CNS hemorrhage in one patient. Fever was the present in 95% of children. Most of the children (65%) were in the high risk group. DIC was present in 90% cases and mean D-Dimer was 4.1 µg /ml. Overall 90% (18/20) patients achieved clinical and peripheral remission with resolution of coagulopathy. But only 75% patients (15/20) reached maintenance therapy in bone marrow remission. Causes of deaths were neutropenic sepsis, intracranial hemorrhage, complicated differentiation syndrome and stroke. Neutropenic sepsis was the most common adverse event. Conclusion: In Bangladeshi pediatric APL patients, neutropenic sepsis is the most common and also the most severe adverse event. Key words: acute promyelocytic leukemia, children, response to treatment